Hearing Loss in Pediatric Patients With Cerebral Palsy.

OBJECTIVE: This study evaluates the prevalence, type, and severity of hearing impairment in children with cerebral palsy (CP) and to analyze audiologic and otologic outcomes in these patients. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGen Database with a diagnosis of cerebral palsy. Appropriate audiologic, otologic, and demographic data were recorded.Nine hundred forty patients met inclusion criteria. Hearing loss (HL) was defined as greater than 15 dB HL at any threshold by pure tone or greater than 20 dB HL by soundfield audiometry. Other available otologic and medical conditions were documented. RESULTS: Of 940 patients, 367 (39%) had hearing loss. Of the 1,629 individual ears with HL, 782 (48%) had conductive, 63 (4%) had sensorineural, 410 (25%) had mixed, and 374 (23%) had unspecified hearing loss. Patients with mixed type 1 hearing loss had significantly worse PTAs. INTERPRETATION: Hearing loss in CP has a large degree of sensorineural loss, with a predisposition to be bilateral. The severity of hearing loss was correlated with the degree of the motor and neurologic disability in these patients.

Otologic and Audiologic Outcomes in Pediatric Patients With Velo-Cardio-Facial (22q11 Deletion) Syndrome.

OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with velo-cardio-facial syndrome (VCFS) and to compare these characteristics with patient demographics and other otologic factors. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGenDB with a diagnosis of velo-cardio-facial syndrome or DiGeorge syndrome. INTERVENTIONS: Appropriate audiologic, otologic, and demographic data were recorded. MAIN OUTCOME MEASURE: Four hundred six patients met inclusion criteria. Data for each patient were selected based on their encounters with earliest complete audiometric data, and hearing loss (HL) was defined as greater than 15 dB HL at any threshold by pure tone or greater than 20 dB HL by soundfield audiometry. The patients were then stratified by type and severity of HL, and available otologic and medical conditions were documented. RESULTS: Two hundred forty eight (60.7%) patients had HL. Of the 391 individual HL ears, 127 had conductive, 22 had sensorineural, 115 had mixed, and 127 had unspecified hearing loss. 57% of the loss was bilateral. Patients with mixed HL had significantly worse pure-tone averages (PTAs). CONCLUSION: HL is prevalent in patients with VCFS. The hearing loss is primarily of conductive origin, with a predisposition to be bilateral, with mixed HL typically have more severe loss. There were a large number of patients with sensorineural hearing loss components as well. Further studies are needed to investigate the causal nature of the hearing impairment in VCFS, to better aid otolaryngologist and audiologists when assessing these patients.

Audiologic Outcomes in Ehlers-Danlos Syndrome.

OBJECTIVE: The focus of this study was to evaluate the prevalence, type, and severity of hearing impairment in patients with Ehlers-Danlos Syndrome (EDS) and to compare these characteristics with patient demographics and other otologic factors. STUDY DESIGN: Retrospective analysis of the AudGen Database. SETTING: Tertiary academic referral center. PATIENTS: Pediatric patients in AudGenDB with a diagnosis EDS. INTERVENTIONS: Appropriate audiologic, otologic, and demographic data were recorded. MAIN OUTCOME MEASURE: One hundred forty one patients met inclusion criteria. Data for each patient were selected based on their first encounter by age with available audiometric data or their first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and available otologic issues were documented. RESULTS: One hundred nine patients had normal hearing. Thirty two patients had hearing loss, with 19 bilateral, and 13 unilateral. Of the 51 individual ears, 25 had conductive, 23 had sensorineural, and 3 had mixed hearing loss. Audiometric configuration was primarily flat in nature. The hearing loss severity was significantly correlated with age, but not influenced by the type of loss. CONCLUSION: Hearing loss is prevalent in patients with EDS. The hearing loss is equally of conductive and sensorineural origin, with a predisposition to be bilateral. Further evaluation of the underlying pathology of hearing loss in these patients is warranted to aid otolaryngologists and audiologists in diagnosis and management.